All of the following statements are true about Wiskott Aldrich syndrome except:
Correct Answer: It is an autosomal recessive disorder
Description: Wiskott-Aldrich syndrome X-linked disease characterized by thrombocytopenia, eczema, and a marked vulnerability to recurrent infection, resulting in early death. There is progressive loss of T-lymphocytes in the peripheral blood and in the T-cell zones (paracoical areas) of the lymph nodes, with variable defects in cellular immunity. Patients do not make antibodies to polysaccharide antigens, and the response to protein antigens is poor. IgM levels in the serum are low, but levels of IgG are usually normal. Paradoxically the levels of IgA and IgE are often elevated. Rx:- Allogeneic hematopoietic stem cell transplant
Category:
Pathology
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